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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
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| {{Infobox Disease | | {{Infobox Disease |
| | Name = Adrenal insufficiency | | | Name = Adrenal insufficiency |
| | Image = Illu adrenal gland.jpg | | | Image = Illu adrenal gland.jpg |
| | Caption = Adrenal gland | | | Caption = Adrenal gland |
| | DiseasesDB =
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| | ICD10 = {{ICD10|E|27|1|e|20}}-{{ICD10|E|27|4|e|20}}
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| | ICD9 = {{ICD9|255.4}}
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| | ICDO =
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| | OMIM =
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| | MedlinePlus =
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| | MeshID = D000309
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| }} | | }} |
| {{SI}} | | {{Secondary adrenal insufficiency}} |
| {{CMG}} | | |
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{SK}} hypocortisolism; | | {{CMG}} {{AE}} {{ADS}} |
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| ==Overview==
| | {{SK}} hypocortisolism; hypocorticism; adrenocortical hypofunction |
| Adrenal insufficiency is a condition in which the [[adrenal gland]]s, located above the [[kidney]]s, do not produce adequate amounts of [[steroid hormone]]s (chemicals produced by the body that regulate organ function), primarily [[cortisol]], but may also include impaired [[aldosterone]] production (a [[mineralocorticoid]]) which regulates [[sodium]], [[potassium]] and water retention.<ref name="addisons_disease.aspx">{{cite journal | author=Eileen K. Corrigan |title=Adrenal Insufficiency (Secondary Addison's or Addison's Disease)|journal=NIH Publication No. 90-3054 |year=2007 |url=http://www.pituitary.org/disorders/addisons_disease.aspx}}</ref><ref>{{MeshName|Adrenal+Insufficiency}}</ref> Craving for salt or salty foods due to the urinary losses of sodium is common.<ref name="PMID 11443143">{{cite journal |author=Ten S, New M, Maclaren N |title=Clinical review 130: Addison's disease 2001 |journal=J. Clin. Endocrinol. Metab. |volume=86 |issue=7 | pages=2909–22 |year=2001 |pmid=11443143 |url=http://jcem.endojournals.org/cgi/content/full/86/7/2909 |doi=10.1210/jc.86.7.2909}}</ref>
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| [[Addison's disease]] and [[congenital adrenal hyperplasia]] can manifest as adrenal insufficiency. If not treated, adrenal insufficiency may result in severe abdominal pains, diarrhea, vomiting, profound muscle weakness and fatigue, [[clinical depression|depression]], extremely low blood pressure ([[hypotension]]), weight loss, kidney failure, changes in mood and personality, and shock (adrenal crisis).<ref name="ch164b.html">{{cite journal | author=Ashley B. Grossman, MD | title=Addison's Disease | journal=Adrenal Gland Disorders | year=2007 | url=http://www.merck.com/mmhe/sec13/ch164/ch164b.html}}</ref> An [[adrenal crisis]] often occurs if the body is subjected to stress, such as an accident, injury, surgery, or severe infection; death may quickly follow.<ref name="ch164b.html" /> | | ==[[Secondary adrenal insufficiency overview|Overview]]== |
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| Adrenal insufficiency can also occur when the [[hypothalamus]] or the [[pituitary gland]], both located at the base of the [[Human skull|skull]], does not make adequate amounts of the hormones that assist in regulating adrenal function.<ref name="addisons_disease.aspx"/><ref name="urlJAMA -- Adrenal Insufficiency, November 16, 2005, Brender et al. 294 (19): 2528">{{cite journal |author=Brender E, Lynm C, Glass RM |title=JAMA patient page. Adrenal insufficiency |journal=JAMA |volume=294 |issue=19 |page=2528 |year=2005 |pmid=16287965 |doi=10.1001/jama.294.19.2528 |url=http://jama.ama-assn.org/cgi/content/full/294/19/2528}}</ref><ref name="urlDorlands Medical Dictionary:adrenal insufficiency">{{cite web |url=http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/four/000053970.htm |title=Dorlands Medical Dictionary:adrenal insufficiency |work= |accessdate=}}</ref> This is called secondary adrenal insufficiency and is caused by lack of production of [[ACTH]] in the pituitary or lack of [[Corticotropin-releasing hormone|CRH]] in the hypothalamus.<ref name="urlSecondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional">{{cite web |url=http://www.merck.com/mmpe/sec12/ch153/ch153c.html |title=Secondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional |work= |accessdate=}}</ref>
| | ==[[Secondary adrenal insufficiency historical perspective|Historical Perspective]]== |
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| ==Classification== | | ==[[Secondary adrenal insufficiency classification|Classification]]== |
| There are three major types of adrenal insufficiency.
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| * ''Primary adrenal insufficiency'' is due to impairment of the adrenal glands.
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| ** One subtype is called [[idiopathic]] or unknown cause of adrenal insufficiency.
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| ** 80% are due to an [[autoimmune disease]] called [[Addison's disease]] or [[autoimmune adrenalitis]].
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| ** Other cases are due to [[congenital adrenal hyperplasia]] or an [[adenoma]] (tumor) of the adrenal gland.
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| * ''Secondary adrenal insufficiency'' is caused by impairment of the [[pituitary gland]] or [[hypothalamus]].<ref name="Hypopituitary">{{cite web |url=http://www.webmd.com/a-to-z-guides/hypopituitary |title=hypopituitary |year=2006}}</ref> The most common cause in the United States is exogenous steroid use. Other causes are a pituitary adenoma or microadenoma, hypothalamic tumor (surgical removal of a pituitary tumor can also suppress production of ACTH and lead to adrenal deficiency); [[Sheehan's syndrome]], which is associated with impairment of only the pituitary gland.
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| * ''Tertiary adrenal insufficiency'' is due to hypothalamic disease and decrease in corticotropin releasing factor (CRF).<ref>http://www.endotext.org/adrenal/adrenal13/adrenal13.htm</ref>
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| == Causes == | | ==[[Secondary adrenal insufficiency pathophysiology|Pathophysiology]]== |
| Causes of acute adrenal insufficiency are mainly [[Waterhouse-Friderichsen syndrome]], sudden withdrawal of long-term [[corticosteroid therapy]] and [[Stress (biology)|stress]] in patients with underlying chronic adrenal insufficiency.<ref name=Robbins20-7>Table 20-7 in: {{cite book |author=Mitchell, Richard Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson |title=Robbins Basic Pathology|publisher=Saunders |location=Philadelphia |year= |pages= |isbn=1-4160-2973-7 |oclc= |doi=}} 8th edition.</ref> The latter is termed [[critical illness–related corticosteroid insufficiency]].
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| For chronic adrenal insufficiency, the major contributors are [[autoimmune adrenalitis]], [[tuberculosis]], [[AIDS]] and [[metastatic]] disease.<ref name=Robbins20-7/> Minor causes of chronic adrenal insufficiency are [[systemic amyloidosis]], [[Fungal infection in animals|fungal infection]]s, [[hemochromatosis]] and [[sarcoidosis]].<ref name=Robbins20-7/>
| | ==[[Secondary adrenal insufficiency causes|Causes]]== |
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| Autoimmune adrenalitis may be part of [[Autoimmune polyendocrine syndrome type 2|Type 2 autoimmune polyglandular syndrome]], which can include [[Diabetes mellitus type 1|type 1 diabetes]], [[hyperthyroidism]], autoimmune [[thyroid disease]] (also known as [[autoimmune thyroiditis]], [[Hashimoto's thyroiditis]] and Hashimoto's disease).<ref name="topic47.htm">{{cite journal | author=Thomas A Wilson, MD | title=Adrenal Insufficiency | journal=Adrenal Gland Disorders|year=2007|url=http://www.emedicine.com/PED/topic47.htm}}</ref> [[Hypogonadism]] and [[pernicious anemia]] may also present with this syndrome.
| | ==[[Differentiating Secondary adrenal insufficiency from other diseases|Differentiating Adrenal Insufficiency from other Diseases]]== |
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| [[Adrenoleukodystrophy]] can also cause adrenal insufficiency.<ref name="921176192.html">{{cite journal | author=Thomas A Wilson, MD | title= Adrenoleukodystrophy |year=1999|url=http://healthlink.mcw.edu/article/921176192.html}}</ref> | | ==[[Secondary adrenal insufficiency epidemiology and demographics|Epidemiology and Demographics]]== |
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| Adrenal Insufficiency can also be caused when a patient has a [[Craniopharyngioma]] which is a benign tumor that can damage the Pituitary gland causing the Adrenal Glands not to function. This would be an example of Secondary Adrenal Insufficiency Syndrome.
| | ==[[Secondary adrenal insufficiency risk factors|Risk Factors]]== |
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| ==Causes== | | ==[[Secondary adrenal insufficiency natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| * '''Acute adrenal insufficiency'''
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| ** [[Addison's disease]] ([[autoimmune adrenalitis]])
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| ** [[Septic shock]]
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| ** [[Waterhouse-Friderichsen syndrome]]
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| ** [[Addison's disease#Addisonian crisis|Addisonian crisis]] in case of:
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| *** Discontinuing [[corticosteroid]] therapy without tapering the dosage
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| *** Surgery, illness or any other form of stress in patients with long-term [[corticosteroid]] therapy
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| * '''Chronic adrenal insufficiency'''
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| ** [[Addison's disease]]
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| ** [[Congenital adrenal hyperplasia|Congenital Adrenal Hyperplasia]]
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| ** [[Antiphospholipid syndrome]]
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| ** [[Haemochromatosis]]
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| ==Diagnosis== | | ==Diagnosis== |
| ===Symptoms===
| | [[Secondary adrenal insufficiency history and symptoms| History and Symptoms]] | [[Secondary adrenal insufficiency physical examination | Physical Examination]] | [[Secondary adrenal insufficiency laboratory findings|Laboratory Findings]] | [[Secondary adrenal insufficiency electrocardiogram|Electrocardiogram]] | [[Secondary adrenal insufficiency chest x ray|Chest X Ray]] | [[Secondary adrenal insufficiency CT|CT]] | [[Secondary adrenal insufficiency MRI|MRI]] | [[Secondary adrenal insufficiency ultrasound|Ultrasound]] | [[Secondary adrenal insufficiency other imaging findings|Other Imaging Findings]] | [[Secondary adrenal insufficiency other diagnostic studies|Other Diagnostic Studies]] |
| There is often the slow insidious onset of the following symptoms (in alphabetical order):
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| *[[Dehydration]]
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| *[[Diarrhea]]
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| *[[Disorientation]]
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| *[[Dizziness]]
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| *[[Hypoglycemia]]
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| *[[Muscle aches]]
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| *[[Nausea]]
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| *[[Tanning]] of the skin that may be patchy or even all over the body. Characteristic sites of tanning are skin creases (e.g. of the hands) and the inside of the cheek (buccal mucosa).
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| *[[Tiredness]]
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| *[[Vomiting]]
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| *[[Weakness]]
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| *[[Weight loss]]
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| ===Physical Examination===
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| ====Vital Signs====
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| [[Hypotension|Low blood pressure]] that falls further when standing ([[orthostatic hypotension]]),
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| [[Cardiovascular collapse]] may be present
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| ====Skin====
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| There can be tanning of the skin.
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| [[Goitre]] and [[vitiligo]] may also be present.<ref name="ch164b.html"/>
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| ===Laboratory Findings===
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| ====ACTH Stimulation Test====
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| If the person is in [[adrenal crisis]], the [[ACTH stimulation test]]<ref>{{cite journal|pmid=21656493 | doi=10.1024/0040-5930/a000174 | volume=68 | issue=6 | title=[Adrenal insufficiency--diagnosis and treatment in clinical practice] | year=2011 | month=June | author=Henzen C | journal=Ther Umsch | pages=337–43}}</ref> may be given. If not in crisis, cortisol, ACTH, aldosterone, renin, potassium and sodium are tested from a blood sample before the decision is made if the ACTH stimulation test needs to be performed. X-rays or CT of the adrenals may also be done.<ref name="addisons_disease.aspx"/> The best test for adrenal insufficiency of autoimmune origin, representing more than ninety percent of all cases in a Western population, is measurement of 21-hydroxylase autoantibodies.
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| {| border="4" cellspacing="1" cellpadding="4" style="text-align:center; margin:10px;"
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| | style="background:#ddcef2" | '''Source of pathology|| style="background:#cef2e0" |'''CRH|| style="background:#cef2e0" |'''ACTH|| style="background:#cef2e0" |'''DHEA|| style="background:#cef2e0" |'''DHEA-S|| style="background:#cef2e0" |'''cortisol||style="background:#cef2e0;" |'''aldosterone|| style="background:#cef2e0" |'''renin|| style="background:#cef2e0" |'''Na|| style="background:#cef2e0" |'''K|| style="background:#f2e0ce" |'''Causes'''<sup>5</sup>
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| | style="background:#cedff2" | '''hypothalamus'''<br>(tertiary)<sup>1</sup>|| style="background:#F8F8F8" |low|| style="background:#F8F8F8"|low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low<sup>3</sup>||style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low||style="background:#F8F8F8" |tumor of the hypothalamus (adenoma), [[antibodies]], [[environment (biophysical)|environment]] (i.e. toxins), [[head injury]]
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| | style="background:#cedff2" | '''pituitary'''<br>(secondary)|| style="background:#F8F8F8" |high<sup>2</sup>|| style="background:#F8F8F8"|low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low<sup>3</sup>||style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low||style="background:#F8F8F8" |tumor of the pituitary ([[adenoma]]), antibodies, environment, head injury,<br>'''surgical removal'''<sup>6</sup>, Sheehan's syndrome
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| | style="background:#cedff2" | '''adrenal glands'''<br>(primary)<sup>7</sup>|| style="background:#F8F8F8" |high|| style="background:#F8F8F8"|high|| style="background:#F8F8F8" |high|| style="background:#F8F8F8" |high|| style="background:#F8F8F8" |low<sup>4</sup>||style="background:#F8F8F8" |low|| style="background:#F8F8F8" |high|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |high||style="background:#F8F8F8" |tumor of the adrenal (adenoma), [[Stress (biology)|stress]], antibodies, environment, [[Addison's Disease]], [[Physical trauma|trauma]], surgical removal ([[Segmental resection|resection]]), [[wikt:miliary|miliary]] [[tuberculosis]] of the adrenal
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| |}
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| {| border="2" cellspacing="1" cellpadding="2" style="margin:10px;"
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| | '''1'''|| Automatically includes diagnosis of secondary (hypopituitarism)
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| | '''2'''|| Only if CRH production in the hypothalamus is intact
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| | '''3'''|| Value doubles or more in stimulation
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| | '''4'''|| Value less than doubles in stimulation
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| | '''5'''|| Most common, does not include all possible causes
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| | '''6'''|| Usually because of very large tumor (macroadenoma)
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| | '''7'''|| Includes Addison's disease
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| |}
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| ==Treatment== | | ==Treatment== |
| *;Adrenal crisis
| | [[Secondary adrenal insufficiency medical therapy|Medical Therapy]] | [[Secondary adrenal insufficiency surgery|Surgery]] | [[Secondary adrenal insufficiency primary prevention|Primary Prevention]] | [[Secondary adrenal insufficiency secondary prevention|Secondary Prevention]] | [[Secondary adrenal insufficiency cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Secondary adrenal insufficiency future or investigational therapies|Future or Investigational Therapies]] |
| **Intravenous fluids<ref name="ch164b.html"/>
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| **Intravenous steroid ([[Solu-Cortef]]/injectable hydrocortisone) later hydrocortisone, prednisone or methylpredisolone tablets<ref name="ch164b.html"/>
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| **Rest
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| *;Cortisol deficiency (primary and secondary)
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| **[[Hydrocortisone]] (Cortef)
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| **[[Prednisone]] (Deltasone)
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| **[[Prednisolone]] (Delta-Cortef)
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| **[[Methylprednisolone]] (Medrol)
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| **[[Dexamethasone]] (Decadron)
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| *;Mineralocorticoid deficiency (low aldosterone)
| | ==Case Studies== |
| **Fludrocortisone Acetate
| | [[Secondary adrenal insufficiency case study one|Case#1]] |
| (To balance sodium, potassium and increase water retention)<ref name="ch164b.html"/>
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| ==See also== | | ==Related Chapters== |
| * [[Addison disease]], primary adrenocortical insufficiency | | * [[Addison disease]], primary adrenocortical insufficiency |
| * [[ACTH stimulation test]] | | * [[ACTH stimulation test]] |
| * [[Cushing's syndrome]], overproduction of cortisol | | * [[Cushing's syndrome]], overproduction of [[cortisol]] |
| * [[Insulin tolerance test]], another test used to identify sub-types of adrenal insufficiency | | * [[Insulin tolerance test]], another test used to identify sub-types of [[adrenal insufficiency]]. |
| * [[Adrenal fatigue]] (hypoadrenia), a term used in [[alternative medicine]] to describe a believed exhaustion of the adrenal glands | | * [[Adrenal fatigue]] (hypoadrenia), a term used in [[alternative medicine]] to describe a believed exhaustion of the [[adrenal glands]]. |
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| ==References==
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| {{reflist|2}}
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| {{Endocrine pathology}} | | {{Endocrine pathology}} |
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| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
| [[Category:Disease]] | | [[Category:Disease]] |
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| [[ca:Insuficiència suprarenal]]
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| [[de:Nebennierenrindeninsuffizienz]]
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| [[es:Insuficiencia adrenal]]
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| [[fr:Insuffisance surrénalienne]]
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| [[he:תת פעילות בלוטת יותרת הכליה]]
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| [[ja:急性副腎不全]]
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| [[ru:Гипокортицизм]]
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